ABSTRACT
Objective:To analyze the frequency and characteristics of polymyositis (PM) in idiopathic inflammatory myopathy (IIM), and to investigate whether PM is over-diagnosed.Methods:Patients diagnosed as IIM according to the Bohan & Peter criteria of IIM hospitalized in the Department of Rheumatology of China-Japan Friendship Hospital from 2008 to 2019 were involved in the study. Definite PM (dPM) was defined as typical clinical and pathological features including elevated creatine kinase (CK) level, muscle weakness and muscle biopsy findings with endomysial CD8 + T cell infiltration and expression of MHC-1 on sarcolemma. Meanwhile, dermatomyositis (DM), anti-synthase syndrome(ASS), immune-mediated necrotic myopathy(IMNM), sporadic inclusion body myositis(sIBM) and other myopathies were excluded according to the new classification criteria of IIM subtypes respectively. Statistical analysis was performed using SPSS software 24.0. The Kruskal-Wallis test and χ2 test were used to compare the clinical characteristics between the dPM group and other IIM subtypes. Results:A total of 1 259 patients with IIM including 1 015 (80.6%) DM and 244(19.4%) PM were enrolled in this study. According to the strict definition of PM criteria, only 0.5% of patients (6/1 259) in IIM could be diagnosed as dPM. Most PM patients were IMNM and ASS according to the new IIM subtypes criteria, of which 48.0% (117/244) were IMNM and 32.0% (78/244) were ASS. 66.7%(4/6) of dPM patients were women. One complicated with RA, and one was dPM overlaped with systemic sclerosis. All of them had muscle weakness, mild elevation of CK level [611(391,1 451) U/L], and were myositis-specific autoantibodies negative. Except one dPM patients who did not receive immunoregulatory therapy due to chronic obstructive pulmonary disease, the others were administrated with low or medium dose prednisone combined with or without immunosuppressive agents. After a median follow-up of (38±26) months, the muscle strength of dPM patients were improved.Conclusion:dPM is a very rare clinical subtype of IIM. PM is an over-diagnosed entity in clinical practice. Patients with dPM have mild symptoms and good outcome.
ABSTRACT
Dyslipidemia is the pathological basis of the occurrence and development of atherosclerosis. It is a major independent risk factor for hypertension, coronary heart disease and cerebrovascular disease. Regulating blood lipid level plays an important role in decreasing the incidence of cardio-cerebrovascular disease. Zhibitai capsule, a lipid-regulating Chinese medicine, has the similar effect to statins. We searched animal experiment studies, clinical trials and reviews in China National Knowledge Infrastructure to analyze the application value and advantages of Zhibitai capsule.
ABSTRACT
Objective:To identify dermatopathological features of patients with dermatomyositis (DM) and analyze its correlation with cutaneous diseases activity.Methods:The clinical data and skin biopsies of 48 patients were collected. The relevance was analyzed using Spearman's correlation analysis. The two groups were compared using Chi-square test or Fisher's exact test. Multi-factors line regression model was established to analyze the relationship between cutaneous disease activity and dermatopathological features.Results:The most common dermatopathological feature was perivascular inflammation (37 cases, 88%), followed by epidermal atrophy (22 cases, 52%) and melanocyte loss (20 cases, 48%), basal vacuolization (15 cases, 36%). The incidence of basal vacuolization ( χ2=9.110, P=0.022), interface dermatitis ( χ2=11.672, P=0.005) and mucin deposition ( χ2=7.795, P=0.029) were significantly different in patients with myositis specific antibody (MSA) subgroup. The patients with positive tranional intermediary factor-1 (anti-TIF1-γ) antibody had higher incidence of interface dermatitis and basal vacuolization, and patients with melanoma differentiation-associated gene 5 (anti-MDA5) antibody had lower incidence of interface dermatitis. Interface dermatitis was positively associated with epidermal atrophy ( r=0.371, P=0.016) and parakeratosis ( r=0.316, P=0.041). Pigment inco-ntinence was positively associated with basal vacuolization ( r=0.384, P=0.012). Multi-factor line regression showed interface dermatitis was positively related to cutaneous disease area and severity index (CDASI). Conclusion:The dermatopathological features is different in subgroup of patients with DM ( β=10.295, P=0.004). Interface dermatitis is a marker of cutaneous disease activity, and its pathogenesis may be different from that of perivascular inflammation. Keratinocytes may be involved in the pathological process in interface dermatitis.
ABSTRACT
Objective:To investigate the pathological characteristics of skeletal muscle and its association with anti-aminoacyl-tRNA synthetases(ARS) antibodies and clinical features in patients with anti-synthetase syndrome (ASS).Methods:Patients diagnosed as ASS at China-Japan Friendship Hospital from 2008 to 2018 were involved in this study. Immunohistochemistry staining of MHC-Ⅰ, CD3, CD4, CD8 and CD20 molecule were performed in all muscle specimen taken from these patients. According to the muscular pathological characteristics, all patients could be divided into six pathological subgroups: pathologic DM(pDM), pathologic PM (pPM), unspecified myositis(USM), necrotizing myopathies (NAM), only MHC-Ⅰexpression (MHC-Ⅰ) and normal pathology groups. Immunoblotting assay was used to detect anti-ARS antibodies. Statistical analysis was performed using Statistical Product and Service Solutions (SPSS) software. T-test, Mann-Whitney U test and Analysis of Variance (ANOVA) were used for the comparison of measurement data. Chi-square test or Fisher's test were used for categorical data. P<0.05 was considered statistically significant. Results:A total of 77 patients underwent muscle biopsy and anti-ARS antibodies test, with the average age of (50±12) years and disease course of 9(3-24) months. The prevalence of anti-Jo-1, anti-PL-7, anti-PL-12, anti-EJ and anti-OJ antibodies in these patients was 47%(36 cases), 29%(22 cases), 12%(9 cases), 13%(10 cases) and 0 respectively. Among all the ASS patients, the most common pathological type was USM(37/77, 48%), followed by pDM(14/77, 18%), the normal pathology(13/77, 17%), NAM(10/77, 13%) and MHC-Ⅰ (3/77, 4%) groups. There were no pPM subtypes in all groups. The frequency of pDM types was significantly different among the anti-ARS antibodies groups( χ2=9.075, P=0.028). The anti-EJ-positive patients had a higher frequency of pDM compared with anti-PL-7-group(40% vs 4%, χ2=6.555, P=0.024). Meanwhile, the CD20 expression in muscle tissue was observed in 30% of anti-EJ-positive patients and 4% of anti-Jo-1-positive ones. There was statistically significant difference in the positive rate of CD20 expression among anti-ARS antibodies groups ( χ2=12.837, P<0.01). Conclusion:The muscle pathological characteristics of ASS are polymorphic and relate to the anti-ARS antibodies. Performing muscle biopsy and distinguishing pathological types are helpful for the diagnosis and stratification of ASS.
ABSTRACT
Objective:To determine the serum levels of chemokine CCL27 and its clinical relevancein patients with dermatomyositis (DM).Methods:The serum CCL27 levels of 58 DM patients, 21 polymyositis (PM) patients, 20 systemic lupus erythematosus (SLE) patients and 31 healthy controls were measured by enzyme linked immunosorbent assay (ELISA). The score of disease activity was measured by two physicians-based on the myositis disease activity assessment tool (MDAAT). Its correlation with serum levels of CCL27 was analyzed. The difference between multiple groups were compared using analysis of variance (ANOVA) and t test, and the relevance was analyzed using Spearman correlation analysis and generalized method of moments (GEE) model. Results:Theserum level of CCL27 in DM patients (178±49) pg/ml was significantly higher than PM (110±40) pg/ml, SLE (141±46) pg/ml and healthy controls (137±38) pg/ml ( F=14.192, P<0.01). Crosssectional analysis showed that the serum CCL27 levelwaspositively correlated with global disease activity ( r=0.301, P=0.022) andskin disease activity ( r=0.493, P<0.01). Patients with V sign had higher serum CCL27 levels (191±52) pg/ml than the patients without (153±33) pg/ml ( t=2.839, P<0.01). Patients with holster sign had higher serum CCL27 levels (196±58) pg/ml than the patients without (168±41) pg/ml ( t=2.176, P=0.034). Follow-up study also found that CCL27l evels were positively correlated with global disease activity ( β=0.031, P=0.042) and skin disease activity ( β=0.032, P<0.01). Conclusion:The serum CCL27 levels are increased in patients with DM and can reflect the skin disease activity. The results of this study suggest that CCL27 may be a marker for cutaneous damage and monitoring of therapeutic effect.
ABSTRACT
Objective To investigate the role of muscle biopsy score in the assessment of the disease activity of idiopathic inflammatory myopathy (IIM) in Chinese adult patients.Methods Total 423 adult IIM patients charged in thedepartment of rheumatology of China-Japan Friendship Hospital from December 2008 to June 2017 were included in this study.The score of disease activity was assessed using International Myositis Assessment and Clinical Studies Group (IMACS) Core Set Measures of IIM.Muscle tissue specimens of IIM patients wasimmunohistochemically stained formajorhistocompatibilitycomplex (MHC)-Ⅰ,CD3 and CD20 molecules.The muscle biopsy score of each specimens was measured separately by four parts of muscle tissue according to muscle fiber,inflammation infiltrating,blood vessel and connective tissue.The correlation between disease activity and muscle biopsy score of IIM was analysed.Statistical analyses were performed using Statistical Product and Service Solutions (SPSS) software.Nonparametric Mann-Wittney U test,Wilcoxon rank sum test and Fisher's exact test were used for between-group comparisons.Nonparametric Spearman's rank correlation was used for correlation analysis.P＜0.05 was considered statistically significant differences.Results The degree of muscle biopsy score was associated with the overall disease activity (r=0.228,P＜0.05),muscle visual analog scale (VAS) (r=0.407,P＜0.05) and the serum levels of creatine kinase (CK) (r=0.466,P＜0.05) of IIM patients,respectively.Among 127 initial patients including 92 dermatoimyositis (DM) and 35polymyositis (PM),the muscle VAS of DM patients were positivelycorrelated to thetotal muscle biopsy score,the score of muscle fiber and inflammation infiltrating (r=0.30,0.312 and 0.241,P＜0.05),respectively.While,the high disease activity of PM patients was related to the increased muscle fiber score (r=0.478,P＜0.05).Conclusion Measurement of muscle biopsy score can be used as an effective tool to evaluate the disease activity of IIM.
ABSTRACT
Objective To investigate the association of distinct myositis specific autoantibodies (MSAs) with long-term survival of patients with polymyositis (PM) and dermatomyositis (DM).Methods We analyzed the clinical data and outcome of patients with PM and DM who were hospita-lized in the department of rheumatology of China-Japan Friendship hospital from 1994 to 2015,and evaluated the impact of MSAs on the prognosis of patients.Multivariate Cox regression analysis was used to identify the prognostic risk factors for PM/DM patients.Results A total of 383 PM/DM patients were followed up for 1-333 months.Cumulative survival and 10-year survival rate of all patients were 68.6％ and 76.2％,respectively.The survival rate of 80.4％ and 77.1％ at 3 and 5 years in patients with MSAs,which were lower than those of patients with-out MSAs,who had the survival rate of 90.1％ and 87.4％ at 3 and 5 years,respectively(x2=3.90 and 3.98,P＜0.05).There was significant difference for long-term survival in all MSAs positive groups (x2=40.654,P=0.000).Anti-MDA5 positive patients who had the 10-year survival rate of 28.7％ had the worst prognosis,while anti-HMGCR positive patients who had the l0-year survival rate of 100％ had the best outcome in all groups.Multivariate Cox regression analysis showed that independent risk factors associated with the long-term survival of patients were age of onset,complicated with malignancies,dysphagia,rapidly progress interstitial lung disease,antiMDA5 antibody positive,increased serum aspartate transferase and C reaction protein.Conclusion MSAs are strongly associated with the prognosis of patients with PM/DM.Patients with MSAs has worse 5-year overall survival than those without MSAs,which indicates that screening MSAs and aggressive treatment for PM/DM patients at very early stage of disease may improve the outcome.
ABSTRACT
Objective To investigate the effect of narrative education on emotional and clinical ability of baccalaureate nursing students practicing in ICU. Methods A total of 42 baccalaureate nursing students practicing in ICU between January 2016 and September 2016 were treated as the control group and received traditional teaching, their counterparts practicing in ICU between October 2016 and June 2017 were served as the observation group (41 cases) and subject to narrative education. The Self-rating Anxiety Scale(SAS),the Self-rating Depression Scale(SDS) were filled out by both groups before and after practicing in ICU, and assessed the comprehensive ability after ICU practice. Results The scores of SAS and SDS after practicing were (49.47 ± 12.37),(50.25 ± 11.86)points in the observation group and (54.32 ± 12.51), (55.79 ± 11.34) points in the control group, and the difference was significant(t=10.822, 12.310, P<0.05). The total scores and personal quality, theoretical knowledge level, clinical nursing ability, judgment thinking ability, communication and cooperation ability was (88.45 ± 13.54), (23.21 ± 4.73), (18.42±3.45), (17.91±3.62), (8.56±0.83), (8.34±0.67) points in the observation group , and (73.13± 11.35), (19.13±4.52), (15.45±3.21), (15.76±3.54), (6.17±0.19), (5.17±0.18) points in the control group, and the difference was significant (t=3.226-8.665, P<0.05 or 0.01). Conlusions The narration education can reduce the psychological burden of baccalaureate nursing students practicing in ICU and can improve the comprehensive ability of them.
ABSTRACT
Objective This study aims to test the hypothesis that low density granulocytes (LDGs) is involved in the pathogenesis of DM associated-Interstitial lung disease (ILD).Methods Forty eight DM patients (28 with ILD) and 19 age-and sex-matched healthy Chinese volunteers were recruited to this study.LDGs percentage in peripheral blood mononuclear cells (PBMCs) was tested by flow cytometry.Neutrophilrelated genes (LL-37,MPO and MMP-8) expressions in PBMCs were tested by quantitative RT-PCR.Myositis disease activity assessment visual analogue scales (MYOACT) was used to assess the disease activity.Percentages of LDGs were compared in patients with ILD and without by using unpaired t test with Welch's correction,the correlations between LDGs and clinical parameters were further analyzed by linear correlation analysis.The expressions of neutrophil-related mRNA and proteins in PBMCs were compared by using MannWhitney U test.Results LDGs percentage in PBMCs was 7.1-fold higher in DM patients than healthy controls [(9.1±11.5)％ vs (1.3±0.7)％,t=4.664,P＜0.01].LDGs percentage in PBMCs was 2.7-fold higher in DM patients with ILD than DM patients without ILD [(12.3±14.1)％ vs (4.5±2.6)％,t=2.835,P=0.008 3].The mRNA expression level of LL-37,MPO and MMP-8 and LL-37 protein levels in the DM group were significantly higher than those in the control group.LDGs percentage positively correlated with MYOACT lung disease activity scores (r=0.439,P=0.010).Conclusion Percentage of LDGs in PBMCs is significantly increased in DM patients with ILD and positively correlated with MYOACT lung disease activity scores,suggesting that abnormall increasing of LDGs is a potential contributor to the pathogenesis of DM-associated ILD.
ABSTRACT
Objective This study was focused on the association between neutrophil extracellular traps (NETs) and interstitial lung disease (ILD) in patients with dermatomyositis (DM).Methods Thirty six patients who satisfied the Bohan & Peter criteria for DM were recruited to this study,among whom 19 were complicated with ILD.Forty seven age and sex matched healthy Chinese volunteers were selected to be control subjects.The plasma samples of these patients were tested for the formation and degradation of NETs.Results DM plasma induced more NETs formation than control plasma did [(246±93) RFUs vs (192±53) RFUs,P=0.002].Compared to control,DM plasma exhibited a signficantry decreased ability to degrade NETs.Further mere,compared with DM patients without ILD (DMNL),DM patients with ILD (DML) could not degrade NETs completely [(83±13)％ vs (59±21)％,P＜0.01].All four DM patients with subacute ILD exhibited a significantly lower ability to degrade NETs than patients with chronic or asymptomatic ILD [(36±14)％ vs (65±19)％,P=0.0139].Conclusion These data show that more NETs formation is induced by plasma and DML fails to completely degrade NETs.These suggest that NETs may play a role in the pathogenesis of DM and DM-associated ILD.
ABSTRACT
Objective To investigate the serum levels of soluble human leukocyte antigen (sHLA)-G in patients with polymyositis (PM) or dermatomyositis (DM),and to analyze its association with clinical features and possible role in the pathogenesis of PM/DM.Methods Serum sHLA-G levels of 26 patients with PM,70 patients with DM and 35 matched healthy controls were measured by ELISA.The relationship between the sHLA-G levels and clinical features or seroimmunological data in the patients with PM/DM was analyzed.Results Serum levels of sHLA-G in PM/DM patients were significantly higher compared to healthy controls [(44±70) U/ml vs (4±5) U/ml,P＜0.01].There was statistically significant difference between DM patients and PM patients [(54±81) U/ml vs (27±41) U/ml,P=0.004].The incidence of dysphagia was significantly higher in sHLA-G elevated group than those in sHLA-G normal group (P=0.001).Additionally,Spearman rank correlation analysis showed that the serum sHLA-G levels were positively correlated with serum C3 (r=0.284,P=0.021),but negatively correlated with CD3+ T cells (r=-0.233,P=0.047) and CD4+ T cells (r=-0.287,P=0.015) in the peripheral blood in patients with PM/DM.Serum levels of sHLA-G in non-treated PM/DM patients were significantly higher compared to treated patients [(77±99) U/ml vs (34±52) U/ml,P=0.021].No relationship between serum sHLA-G levels and PM/DM disease activity,or different drug therapy was found.Conclusion Serum levels of sHLA-G are increased in PM/DM patients.The increased production of sHLA-G,paralleled with higher incidence of dysphagia and lower level of CD3+ T cells and CD4+ T cells,indicates that sHLA-G may play an important role in the pathogenesis of PM/DM.
ABSTRACT
ObjectiveTo investigate the clinical significance of peripheral blood lymphocyte subsets in patients with idiopathic inflammatory myopathy (ⅡM).MethodsPeripheral blood lymphocyte subsets was determined by flow cytometry in 89 patients with polymyositis(PM ) or dermatomyositis ( DM ).The association between clinical features and peripheral blood lymphocyte subsets was analyzed by F test,t test and x2 test.ResultsPatients with active DM showed significant decreases in counts of CD3+ cell,CD3+CD4+ cell and CD3+CD8+ cell[(8±4),(5.4±2.8) and (2.6±1.6) ×108/L respectively],compared with those in inactive DM [(16±6), (10.4±5.6) and (5.6±3.8) ×108/L respectively] and healthy controls [(14±4), (8.3±2.8) and (4.6±1.7) ×108/Lrespectively](F=12.901,8.257,7.084; P＜0.05).Logistic regression analysis indicated that myositis disease activity could influence the counts of peripheral blood CD3+ cell,CD3+CD4+ cell and CD3+ CD8+ cell (b=0.211,0.344,0.289; P＜0.05 ).ILD in ⅡM could influence the counts of CD3+ cell and the ratio of CD3+CD4+cell (b=0.928,1.974; P＜0.05 ).Logistic regression analysis indicated that the count of CD3+CD8+ cell was risk factor for death,and the relative risk was 0.989(b=-0.011 ; P＜0.05).ConclusionPeripheral blood lymphocyte subsets may be regarded as useful laboratory parameters for monitoring RA disease activity.Decreased CD8+ T cell may predict a poor outcome of patients with IIM.